Successful Coil Embolization of a Ruptured Basilar Artery Aneurysm in a Child with Leukemia: A Case Report

Ruptured intracranial aneurysms are rare in the pediatric population compared to adults. This has incited considerable discussion on how to treat children with this condition. Here, we report a child with a ruptured saccular basilar artery aneurysm that was successfully treated with coil embolization. A 12-year-old boy with acute lymphoblastic leukemia and accompanying abdominal candidiasis after chemotherapy suddenly complained of a severe headache and suffered consciousness disturbance moments later. Computed tomography scans and cerebral angiography demonstrated acute hydrocephalus and subarachnoid hemorrhage caused by saccular basilar artery aneurysm rupture. External ventricular drainage was performed immediately. Because the patient was in severe condition and did not show remarkable signs of central nervous system infection in cerebrospinal fluid studies, we applied endovascular treatment for the ruptured saccular basilar artery aneurysm, which was successfully occluded with coils. The patient recovered without new neurological deficits after ventriculoperitoneal shunting. Recent reports indicate that both endovascular and microsurgical techniques can be used to effectively treat ruptured cerebral aneurysms in pediatric patients. A minimally invasive endovascular treatment was effective in the present case, but long-term follow-up will be necessary to confirm the efficiency of endovascular treatment for children with ruptured saccular basilar artery aneurysms.     

Nutrition‐related factors associated with waiting list mortality in patients with interstitial lung disease: A retrospective cohort study

Japanese patients with interstitial lung disease (ILD) sometimes die waiting for lung transplantation (LTx) because it takes about 2 years to receive it in Japan. We evaluated nutrition‐related factors associated with waiting list mortality. Seventy‐six ILD patients were hospitalized in Kyoto University Hospital at registration for LTx from 2013 to 2015. Among them, 40 patients were included and analyzed. Patient background was as follows: female, 30%; age, 50.3 ± 6.9 years; body mass index, 21.1 ± 4.0 kg/m²; 6‐minute walk distance (6MWD), 356 ± 172 m; serum albumin, 3.8 ± 0.4 g/dL; serum transthyretin (TTR), 25.3 ± 7.5 mg/dL; and C‐reactive protein, 0.5 ± 0.5 mg/dL. Median observational period was 497 (range 97‐1015) days, and median survival time was 550 (95% CI 414‐686) days. Survival rate was 47.5%, and mortality rate was 38.7/100 person‐years. Cox analyses showed that TTR (HR 0.791, 95% CI 0.633‐0.988) and 6MWD (HR 0.795, 95% CI 0.674‐0.938) were independently correlated with mortality and were influenced by body fat mass and leg skeletal muscle mass, respectively. It is suggested that nutritional markers and exercise capacity are important prognostic markers in waitlisted patients, but further study is needed to determine whether nutritional intervention or exercise can change outcomes.     

Four novel mutations in the thiazide-sensitive Na-Cl co-transporter gene in Japanese patients with Gitelman's syndrome.

BACKGROUND: Gitelman's syndrome (GS) is an autosomal recessive disorder resulting from inactivating mutations in the thiazide-sensitive Na-Cl co-transporter (NCCT) gene. To date, almost 90 mutations have been identified. It is possible that there is a population-specific distribution of mutations. In this study, we analysed mutations in the NCCT gene of seven Japanese patients with GS. METHODS: Peripheral blood mononuclear cells were isolated from patients with GS, their family members and healthy control subjects. A mutation analysis of the NCCT gene was performed completely by direct automated sequencing of polymerase chain reaction-amplified DNA products. In patients with a deletion or splice site mutation, we undertook cDNA sequence analysis. RESULTS: We identified nine mutations. Five of them [c.185C>T (Thr60Met), c.1712C>T (Ala569Val), c.1930C>T (Arg642Cys), c.2552T>A (Leu849His) and c.1932delC] have been reported in Japanese patients, but not in GS patients from other ethnic groups. The remaining four mutations [c.7A>T (Met1Leu), c.1181_1186+20del26, c.1811_1812delAT and IVS16+1G>A] were novel. In cDNA derived from a patient with c.1181_1186+20del26, a deletion of exon 9 and a frameshift at the start of exon 10 were observed. In cDNA derived from patients with IVS16+1G>A, an additional 96 bp insertion between exons 16 and 17 was observed. Six out of seven patients were compound heterozygotes, and the remaining one carried a single heterozygous mutation. CONCLUSIONS: We found four novel mutations in the NCCT gene in seven Japanese patients with GS. Moreover, our study suggests that the distribution of mutations in the NCCT gene in Japanese GS patients potentially differs from that in other populations.     

A case of minimal change nephrotic syndrome with immunoglobulin A nephropathy transitioned to focal segmental glomerulosclerosis

A 50-year-old woman with a 1-month history of lower extremity edema and a 5 kg weight increase was admitted to our hospital with suspected nephrotic syndrome in October 1999. Urine protein level was 3.5 g per day, 10-15 erythrocytes in urine per high-power field, and serum albumin level 2.5 g/dl. Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray. The results of a renal biopsy indicated slight mesangial proliferation in the glomeruli by light microscopy, and an immunofluorescence study confirmed the deposition of immunoglobulin (Ig) A and C3 in the mesangial area. Diffuse attenuation of foot processes and dense deposits in the mesangial area were observed by electron microscopy. Treatment with 40 mg/day of prednisolone was effective, and proteinuria was negative 1 month later. Because of this course, we diagnosed minimal change nephrotic syndrome complicated by mild-proliferative IgA nephropathy. In November 2000, there was a relapse of nephrotic syndrome, which was believed to be induced by an influenza vaccination, but response to increased steroid treatment was favorable, and proteinuria disappeared on day 13 of steroid increase. A second relapse in May 2001, showed steroid resistance with renal insufficiency, and an increase in the selectivity index to 0.195. Light microscopy revealed focal sclerotic lesions of the glomeruli, and an immunofluorescence study revealed attenuation of mesangial IgA and C3 deposition. These findings led to the diagnosis that minimal change nephrotic syndrome had transitioned to focal segmental glomerulosclerosis, whereby mesangial IgA deposition was reduced by immunosuppressive treatment. Subsequently, her renal function gradually worsened to the point of end-stage renal failure by 27 months after the second relapse of nephrotic syndrome.     

Three-dimensional transesophageal echocardiography in the diagnosis of atrial septal aneurysm with patent foramen ovale in a patient with ventricular septal defect

We present a case of a rare combination of ventricular septal defect and atrial septal aneurysm (ASA) with a patent foramen ovale (PFO) in a 57-year-old female. She was referred to our hospital for the treatment of irregular palpitation and exertional shortness of breath. Two-dimensional transesophageal echocardiography (2D-TEE) with color Doppler imaging demonstrated an ASA with two lines of shunt flows. Three-dimensional transesophageal echocardiography (3D-TEE) could clearly visualize the morphology of the PFO in the surgeon??s view and demonstrated a crescent-shaped opening of the PFO. 3D-TEE appears to be a more accurate modality than 2D-TEE for the assessment of PFO.     

Pineal xanthogranuloma

A 64-year-old man presented a large pineal cystic lesion manifesting as headache and exhibiting unusual neuroradiological findings. Magnetic resonance imaging showed a cystic lesion appearing as hyperintense on both T1- and T2-weighted images, and a nodular lesion which was hypointense on T1-and mixed intensity on T2-weighted images. The cystic mass was removed via a right occipital transtentorial approach. Histological examination disclosed that the inner surface of the cystic part consisted of bi-layered epithelial lining, portions of which had changed to stratified squamous epithelium. The solid part showed the characteristics of xanthogranuloma such as cholesterol clefts, hemosiderin-laden macrophages, and foreign body giant cells.     

External pneumatic compression does not increase urokinase plasminogen activator after abdominal surgery

External pneumatic compression (EPC) devices prevent lower extremity deep venous thrombosis (DVT) by reducing stasis. There is a widely held belief that they also enhance endogenous fibrinolysis; however, recent studies of tissue plasminogen activator (the primary activator of fibrinolysis) and plasminogen activator inhibitor-1 (the primary inhibitor of fibrinolysis) failed to confirm this. The hypothesis of this study was that EPC devices increase the level of urokinase plasminogen activator (uPA), a second activator of fibrinolysis. This was a prospective trial in which 44 subjects who underwent major abdominal surgery were randomized to receive unfractionated heparin injections, thigh-length sequential EPC devices, or both for DVT prophylaxis. Prophylaxis was begun immediately before surgical incision and continued until postoperative day 5 or discharge. Venous blood samples were collected from an antecubital vein for measurement of systemic uPA levels and from the common femoral vein for measurement of regional uPA levels. Samples were collected the day before surgery, after induction of anesthesia but before surgical incision, and on postoperative days 1, 3, and 5. uPA levels (ng/mL) were measured with an enzyme-linked immunoassay. Baseline uPA levels (0.41 to 0.56 ng/mL; P >.05, analysis of variance with repeated measures) were similar among the three groups. uPA levels did not change after surgery in systemic or regional blood samples in any group. There were no significant differences in systemic or regional uPA levels in the groups treated with EPC devices relative to those treated with heparin at any time point (P >.05, analysis of variance with repeated measures). Enhancement of fibrinolysis with EPC devices remains unproven; the findings reported here suggest that effective DVT prophylaxis can only be assured when the devices are used in a manner that reduces venous stasis.     

Case report of post-traumatic arterial high-flow priapism

Priapism is rare and usually unpredictable. High-flow priapism is caused by unregulated arterial inflow. Antecedent trauma is the most commonly described etiology. This condition does not require emergent treatment. The initial management of high-flow priapism should be observation, because treatment-related erectile dysfunction may appear. We report a case of high-flow priapism by perineal trauma in a 27-year-old man. His corpora were typically tumescent, but not completely rigid. He could not have sexual intercourse. Blood from the corpus cavernosum was normally oxygenated. Color duplex ultrasonography was performed in the lithotomy position, scanned at the perineum, showed pseudoaneurysmal appearance. Selective internal pudendal arteriography showed a right cavernous arterial extravasation. Superselective embolization of right internal pudendal arteries was performed with an autologous clot. After the procedure, detumescence was achieved as well as erectile function. We recommend superselective arterial embolization as the management of high flow priapism to patients who request treatment.     

Reconstruction methods to achieve optimal postoperative bowel function following low anterior resection for rectal cancer

After low anterior resection for rectal cancer, approximately 50% of patients experience defecatory malfunction such as multiple evacuations, urgency, and soiling. Since the neorectum is constructed with the remaining colonic segment, it can only substitute for the rectum to a limited extent. A straight anastomosis is most frequently used when the rectal remnant is sufficient, such as in high anterior resection. When the height of anastomosis is close to the anal sphincter, a J-pouch, a side-to-end, or a transverse coloplasty pouch are constructed to achieve better postoperative bowel function. The advantage of J-pouch reconstruction is not only the increased volume but also may be decreased motility when compared with straight reconstruction. In terms of postoperative function, the side-to-end and transverse coloplasty pouch have both been reported to exhibit similar functional results to J-pouch reconstruction. To obtain optimal functional results, pouch reconstruction should be considered, especially when the height of anastomosis is at the levator plane.     

Neural correlates of true memory, false memory, and deception

We used functional magnetic resonance imaging (fMRI) to determine whether neural activity can differentiate between true memory, false memory, and deception. Subjects heard a series of semantically related words and were later asked to make a recognition judgment of old words, semantically related nonstudied words (lures for false recognition), and unrelated new words. They were also asked to make a deceptive response to half of the old and unrelated new words. There were 3 main findings. First, consistent with the notion that executive function supports deception, 2 types of deception (pretending to know and pretending not to know) recruited prefrontal activity. Second, consistent with the sensory reactivation hypothesis, the difference between true recognition and false recognition was found in the left temporoparietal regions probably engaged in the encoding of auditorily presented words. Third, the left prefrontal cortex was activated during pretending to know relative to correct rejection and false recognition, whereas the right anterior hippocampus was activated during false recognition relative to correct rejection and pretending to know. These findings indicate that fMRI can detect the difference in brain activity between deception and false memory despite the fact that subjects respond with "I know" to novel events in both processes.